Anti-phospholipid antibodies (aPL) are the serological biomarkers of anti-phospholipid syndrome (APS), an autoimmune disorder characterized by vascular events and/or pregnancy morbidity. APS is a unique condition as thrombosis might occur in arterial, venous or capillary circulations. The heart provides a frequent target for circulating aPL, leading to a wide variety of clinical manifestations. The most common cardiac presentation in APS, valvular involvement, acknowledges a dual etiology comprising both microthrombotic and inflammatory mechanisms. We describe the cases of 4 patients with primary APS who presented a clinically manifest myocardiopathy without epicardial macrovascular distribution. We propose that microthrombotic/inflammatory myocardiopathy might be an overlooked complication of high-risk APS. As extensively hereby reviewed, the literature provides support to this hypothesis in terms of anecdotal case-reports, in some cases with myocardial bioptic specimens. In aPL-positive subjects, microthrombotic/inflammatory myocardial involvement might also clinically manifest as dilated cardiomyopathy, a clinical entity characterized by ventricular dilation and reduced cardiac output. Furthermore, microthrombotic/inflammatory myocardial involvement might be subclinical, presenting as diastolic dysfunction. Currently, there is no single clinical or imaging finding to firmly confirm the diagnosis; an integrated approach including clinical history, clinical assessment, laboratory tests and cardiac magnetic resonance should be pursued in patients with suggestive clinical presentation.

Myocardial involvement in anti-phospholipid syndrome: Beyond acute myocardial infarction / L. Coletto, M. Gerosa, M. Valentini, R. Cimaz, R. Caporali, P. Meroni, C. Chighizola. - In: AUTOIMMUNITY REVIEWS. - ISSN 1568-9972. - 21:3(2022 Mar), pp. 102990.1-102990.7. [10.1016/j.autrev.2021.102990]

Myocardial involvement in anti-phospholipid syndrome: Beyond acute myocardial infarction

L. Coletto;M. Gerosa
;
R. Cimaz;R. Caporali;P. Meroni;C. Chighizola
2022-03

Abstract

Anti-phospholipid antibodies (aPL) are the serological biomarkers of anti-phospholipid syndrome (APS), an autoimmune disorder characterized by vascular events and/or pregnancy morbidity. APS is a unique condition as thrombosis might occur in arterial, venous or capillary circulations. The heart provides a frequent target for circulating aPL, leading to a wide variety of clinical manifestations. The most common cardiac presentation in APS, valvular involvement, acknowledges a dual etiology comprising both microthrombotic and inflammatory mechanisms. We describe the cases of 4 patients with primary APS who presented a clinically manifest myocardiopathy without epicardial macrovascular distribution. We propose that microthrombotic/inflammatory myocardiopathy might be an overlooked complication of high-risk APS. As extensively hereby reviewed, the literature provides support to this hypothesis in terms of anecdotal case-reports, in some cases with myocardial bioptic specimens. In aPL-positive subjects, microthrombotic/inflammatory myocardial involvement might also clinically manifest as dilated cardiomyopathy, a clinical entity characterized by ventricular dilation and reduced cardiac output. Furthermore, microthrombotic/inflammatory myocardial involvement might be subclinical, presenting as diastolic dysfunction. Currently, there is no single clinical or imaging finding to firmly confirm the diagnosis; an integrated approach including clinical history, clinical assessment, laboratory tests and cardiac magnetic resonance should be pursued in patients with suggestive clinical presentation.
Anti-phospholipid antibodies; Heart involvement; Myocardium; Thrombosis; Inflammation; Systolic dysfunction; Dilated cardiomyopathy;
Settore MED/16 - Reumatologia
2-nov-2921
Article (author)
File in questo prodotto:
File Dimensione Formato  
Coletto Autoimmun Rev.pdf

accesso riservato

Tipologia: Publisher's version/PDF
Dimensione 441.83 kB
Formato Adobe PDF
441.83 kB Adobe PDF   Visualizza/Apri   Richiedi una copia
Pubblicazioni consigliate

Caricamento pubblicazioni consigliate

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/2434/905628
Citazioni
  • ???jsp.display-item.citation.pmc??? 0
  • Scopus 0
  • ???jsp.display-item.citation.isi??? ND
social impact